Several challenges associated with the anesthetic management of Emery-Dreifuss muscular dystrophy patients: case report

Abstract Emery-Dreifuss Muscular Dystrophy is a very rare type of muscular dystrophy, associated with contractures, atrophy, and muscle weakness, besides cardiomyopathy with severe arrhythmias. Published studies focusing on this disorder are scarce. We describe the anesthetic management of a male patient with Emery-Dreifuss Muscular Dystrophy, to be submitted to umbilical and inguinal hernioplasty and hydrocele repair under epidural anesthesia. The anesthesia approach enabled us to circumvent the patient's susceptibility to malignant hyperthermia and his potentially difficult airway, in addition to maintaining hemodynamic stability. The day after surgery the patient resumed walking, and two days later he was discharged from the hospital.

Several challenges associated with the anesthetic management of Emery-Dreifuss muscular dystrophy patients: case report.

Emery-Dreifuss Muscular Dystrophy is a very rare type of muscular dystrophy, associated with contractures, atrophy, and muscle weakness, besides cardiomyopathy with severe arrhythmias. Published studies focusing on this disorder are scarce. We describe the anesthetic management of a male patient with Emery-Dreifuss Muscular Dystrophy, to be submitted to umbilical and inguinal hernioplasty and hydrocele repair under epidural anesthesia. The anesthesia approach enabled us to circumvent the patient...s susceptibility to malignant hyperthermia and his potentially difficult airway, in addition to maintaining hemodynamic stability. The day after surgery the patient resumed walking, and two days later he was discharged from the hospital.

Approach and anesthetic management for kidney transplantation in a patient with bilateral lung transplantation: case report

Abstract Lung transplantation is the last resort for end-stage lung disease treatment. Due to increased survival, lung recipients present an increased likelihood to be submitted to anesthesia and surgery. This case report describes a 23-year-old female patient with history of lung transplantation for cystic fibrosis, with multiple complications, and chronic kidney disease, and who underwent kidney transplantation under general anesthesia. Understanding the pathophysiology and changes related to immunosuppressive therapy is essential to anesthetic technique planning and safety, and for perioperative management. The success of both anesthesia and surgery requires a qualified multidisciplinary team due to the rarity of the clinical scenario and high incidence of associated morbidity and mortality.

Obstetric Admissions to the Intensive Care Unit: A 18-Year Review in a Portuguese Tertiary Care Centre.

INTRODUCTION: Maternal mortality and morbidity are important indicators of the quality of health-care services. Obstetric admissions to an intensive care unit may be considered a marker of maternal morbidity. The aim of this study was to determine the incidence, maternal morbidity and mortality of pregnant and postpartum women who required admission to the intensive care unit. MATERIAL AND METHODS: Retrospective analysis of all the obstetric patients admitted to the intensive care unit between 2000 and 2017. Results: Ninety-three women required admission to intensive care (0.7 per 1000 deliveries, 0.8% of all adult admissions). Mean age was 30.3 years, mean gestational age was 33.6 weeks, 51 (54.8%) were primiparous, nine (9.7%) were pregnant of twins and five (5.4%) had not been followed during pregnancy. Eighty-four (90.3%) were admitted after immediate delivery. The most common reasons for admission were hypertensive disorders of pregnancy (35.5%) and obstetric haemorrhage (24.7%). Median length of stay was five days. Transfusion of blood products was needed in 23 (57.0%), artificial ventilation in 50 (53.8%) and use of vasopressors in 21 (22.6%). We observed four maternal deaths (4.3%). Most patients (95.7%) successfully recovered and were transferred to other departments. Sequential Organ Failure Assessment score was significantly associated with maternal mortality. DISCUSSION: Our results are comparable to those obtained in other studies. Maternal mortality was comparable to maternal mortality in developed countries. CONCLUSION: The incidence of obstetric admissions to the intensive care unit was 0.8% and 0.7 per 1000 deliveries. Hypertensive disorders of pregnancy were the main causes of admission. Maternal mortality was 4.3%. Studies of maternal morbidity are important and can help to improve the quality of health care services.

Introdução: Mortalidade e morbilidade maternas são importantes indicadores da qualidade dos cuidados de saúde. As admissões obstétricas numa unidade de cuidados Intensivos podem ser consideradas um marcador de morbilidade materna. O objetivo deste estudo foi determinar a incidência, morbilidade e mortalidade maternas das gestantes e puérperas admitidas na unidade de cuidados intensivos. Material e Métodos: Estudo retrospetivo de todas as admissões obstétricas na unidade de cuidados intensivos no período de 2000 a 2017. Resultados: Noventa e três mulheres necessitaram de internamento em Cuidados Intensivos (0,7 por 1000 partos e 0,8% de todas as admissões de adultos). A média de idades foi de 30,3 anos, a idade gestacional média foi 33,6 semanas, 51 (54,8%) eram primíparas, nove (9,7%) eram gravidezes gemelares e cinco (5,4%) não tinham sido vigiadas durante a gravidez. Oitenta e quatro (90,3%) foram admitidas no período pós-parto imediato. Os motivos mais comuns de internamento foram os distúrbios hipertensivos da gravidez (35,5%) e hemorragia obstétrica (24,7%). A mediana do tempo de internamento foi de cinco dias. A transfusão de hemoderivados foi necessária em 53 (57,0%), ventilação artificial em 50 (53,8%) e uso de vasopressores em 21 (22,6%). Observámos quatro óbitos maternos (4,3%). A maioria das doentes (95,7%) recuperaram e foram transferidas para outros serviços. O valor do Sequential Organ Failure Assessment score teve associação estaticamente significativa com a mortalidade materna. Discussão: Os resultados obtidos são comparáveis aos obtidos em outros estudos. A mortalidade materna foi semelhante à mortalidade materna nos países desenvolvidos. Conclusão: A incidência de admissões obstétricas numa unidade de cuidados intensivos foi de 0,8% e 0,7 por 1000 partos. Os distúrbios hipertensivos da gravidez foram as principais causas de internamento. A mortalidade materna foi de 4,3%. Estudos de morbilidade materna são importantes e podem ajudar a melhorar a qualidade dos serviços de saúde.